Torsades de pointes short-coupling

BACKGROUND: The short-coupled variant of torsade de pointes (sc-TdP) is a malignant arrhythmia that frequently presents with ventricular fibrillation (VF) electrical storm. Verapamil is considered the first-line therapy of sc-TdP while catheter ablation is not widely adopted. The aim of this study was to determine the origin of sc-TdP and to assess the outcome of catheter ablation using 3D-mapping. METHODS AND RESULTS: We retrospectively analyzed five patients with sc-TdP who underwent 3D-mapping and ablation of sc-TdP at five different institutions...

AIMS: Distinctive types of polymorphic ventricular tachycardia (VT) respond differently to different forms of therapy. We therefore performed the present study to define the electrocardiographic characteristics of different forms of polymorphic VT. METHODS AND RESULTS: We studied 190 patients for whom the onset of 305 polymorphic VT events was available. The study group included 87 patients with coronary artery disease who had spontaneous polymorphic VT triggered by short-coupled extrasystoles in the absence of myocardial ischaemia...

Idiopathic ventricular fibrillation (IVF) causes sudden death in young adult patients without structural or ischemic heart disease. Most IVF cases are sporadic and some patients present with short-coupled torsade de pointes, the genetics of which are poorly understood. A man who had a first syncope at the age of 35 presented with frequent short-coupled premature ventricular beats with bursts of polymorphic ventricular tachycardia and then died suddenly. By exome sequencing, we identified three rare variants: p...

In this case report, we describe a 73 year old female with structuraly normal heart that developed shortcoupled torsades de pointes (TdP) resulting in an electrical storm unresponsible to several antiarrhythmic drugs, but fully controlled with verapamil. The critical timing of the ventricular premature beats that initiated TdP corresponded to those that occurred at the peak of the previous T wave. This behavior differentiates this entity from other forms of malignant ventricular arrhythmias in patients with structurally normal heart...

BACKGROUND: Torsade de Pointes arrhythmia is a potentially lethal polymorphic ventricular tachyarrhythmia (pVT) in the setting of long QT syndrome. Arrhythmia susceptibility is influenced by risk factors modifying repolarization. OBJECTIVE: To characterize repolarization duration and heterogeneity in relation to pVT inducibility and maintenance. METHODS: Sotalol was infused regionally or globally in isolated Langendorff blood-perfused pig hearts (N=7) to create repolarization time (RT) heterogeneities...

Idiopathic ventricular fibrillation (IVF) is a diagnosis of exclusion made when no underlying cause is identified in a cardiac arrest survivor. Although the frequency of this diagnosis has declined over time due to advances in diagnostic techniques, it remains a substantial cause of sudden cardiac arrest. Further, IVF tends to recur. This article reviews the criteria for diagnosis, patient characteristics, the two primary arrhythmic phenotypes-short-coupled variant of torsades de pointes and recurrent paroxysmal IVF-and the electrophysiologic features, treatment, and ablation of premature ventricular complexes that can trigger IVF...

BACKGROUND: Short-coupled variant of torsades de pointes (scTdP) is a disease characterized by TdP without QT prolongation, which is initiated by extremely short-coupled ventricular extra-systoles. Its genetic background remains rarely unveiled. OBJECTIVE: We aimed to identify genetic variations in patients with scTdP, and to analyze the functional change of the mutant Na+ channel identified in a scTdP patient. METHODS AND RESULTS: We performed genetic analysis for inherited arrhythmia-related 45 genes using next-generation sequencer (MiSeq, Illumina) among 7 consecutive scTdP patients...

A 40-year-old male visited our institute complaining of transient loss of consciousness. He had been implanted with an implantable cardioverter defibrillator (ICD) due to idiopathic ventricular fibrillation for secondary prevention. His past genetic screening detected a single nucleotide SCN5A mutation (pR18Q), while neither QT prolongation nor ST segment elevation in the right precordial leads was observed. An interrogation of the ICD revealed that a shock therapy successfully terminated ventricular fibrillation at the time syncope occurred...

Introduction : Ventricular arrhythmias are often seen in association with structural heart disease. However, approximately a tenth of affected patients have apparently normal hearts, where such arrhythmias typically occur in young patients, are sometimes inherited and can occasionally lead to sudden cardiac death (SCD). Over the past two decades, increased understanding of the underlying pathophysiology resulted in improved targeted pharmacological therapy. Areas covered : This article reviews current knowledge regarding drug therapy for inherited arrhythmia syndromes (Brugada, early repolarization, long QT and short QT syndromes, and catecholaminergic polymorphic ventricular tachycardia), and acquired arrhythmias (idiopathic ventricular fibrillation, short-coupled torsade de pointes, outflow tract ventricular tachycardia, idiopathic left, papillary muscle and annular ventricular tachycardias)...

BACKGROUND: A short-coupled variant of torsade de pointes (ScTdP) is rare and resistant to medical treatment. There has not been a reported catheter ablation (CA) of a short-coupled premature ventricular contraction (PVC) triggering ScTdP in an infant. CASE SUMMARY: A neonate was referred to our hospital on the day of birth for Wolff-Parkinson-White syndrome, repeated episodes of supraventricular tachycardia, and a left ventricular non-compaction. She underwent CA of an accessory pathway at 72 days of age...

A 44-year-old woman with previous myocarditis underwent several syncopal events due to self-terminated ventricular fibrillation (VF) with hypokalemia. Electrocardiogram showed a normal QT duration and premature ventricular contraction with a short coupling interval (280 ms), and the R-on-T phenomenon induced Torsade de pointes (TdP) that deteriorated into VF, a condition known as short coupled TdP (ScTdP). Cardiac magnetic resonance imaging showed left ventricular dilatation and diffuse high T2 signal intensity, and endomyocardial biopsy exhibited a failing myocardium...

Introduction: Idiopathic short-coupled ventricular tachyarrhythmias make up a considerable proportion of ventricular tachyarrhythmias in structurally normal hearts and are the cause of 5-10% of unexpected sudden cardiac deaths. There is disparity in the literature regarding their description and a lack of formal diagnostic criteria to define them. Objective: To validate ECG indices for the diagnosis of these ventricular tachyarrythmias and to subsequently unify their differing descriptions in the literature under a new terminology: Idiopathic Short-Coupled Ventricular Tachyarrhythmias ...

A 10-month-old infant experienced cardiac arrest caused by ventricular fibrillation (VF). His electrocardiogram (ECG) at rest was within the normal range. Amiodarone was indispensable due to its refractoriness to defibrillation. After implantable cardioverter defibrillator (ICD) implantation, ICD shock was delivered. ICD recordings documented VF and ventricular tachycardia (VT) triggered by premature ventricular contractions with an extremely short coupling interval (240 ms), which were controlled by verapamil...

We present a case of sudden cardiac arrest 7 hours after radiofrequency ablation of the atrioventricular junction for symptomatic permanent atrial fibrillation unresponsive to medical therapy. The Holter monitoring revealed a progressive increasing of QT interval after the procedure, highlighting the repolarization instability after acute changes in heart rates associated with modification of ventricular activation, leading to occurrence of short coupling interval ventricular extra beats and finally to a "torsade de pointes...

BACKGROUND: Ventricular fibrillation may be caused by premature ventricular contractions (PVCs) whose coupling intervals are <300 ms, a characteristic of the short-coupled variant of torsades de pointes (scTdP). OBJECTIVE: The purpose of this study was to analyze the underlying cardiac ryanodine receptor (RyR2) variants in patients with scTdP. METHODS: Seven patients with scTdP (mean age 34 ± 12 years; 4 men and 3 women) were enrolled in this study...

A 49-year-old woman, with no relevant family history, was admitted in 1996 for arrhythmic storm with polymorphic ventricular tachycardia (torsade de pointes) which degenerated into ventricular fibrillation. Iatrogenic causes were excluded, the electrocardiogram (ECG) was normal and there was no structural heart disease. She refused cardioverter-defibrillator implantation. Treatment was begun with amiodarone, which she took irregularly. She remained asymptomatic until 2014 when she was admitted for a new arrhythmic storm with torsade de pointes, refractory to antiarrhythmic therapy and aggravated by ventricular pacing (65 defibrillations)...

BACKGROUND: Acquired long QT syndrome (a-LQTS) is associated with life-threatening ventricular arrhythmias, mainly torsades de pointes (TdP). ECG parameters predicting evolving into ventricular fibrillation (VF) are ill defined. AIMS: To determine ECG parameters preceding and during TdP associated with higher risk of developing VF. METHODS: We analyzed 151 episodes of TdP, recorded in 28 patients with a-LQTS (mean QTc 638 ms ± 57). RESULTS: All 28 patients had prolonged QT interval, (mean QTc 638 ms ± 57) ranging from 502 ms to 858 ms correcting by Bazett's formula...

The polymorphic ventricular tachycardia (PVT) is uncommon arrhythmia with multiple causes and has been classified according to whether they are associated with long QT interval or normal QT. Whereas "Torsade de pointes (TdP)" is an uncommon and distinctive form of PVT occurring in a setting of prolonged QT interval, which may be congenital or acquired (congenital or acquired), "PVT with normal QT" is associated with myocardial ischemia, electrolyte abnormalities (hypokalemia), mutations of the cardiac sodium channel (Brugada syndrome), and the ryanodine receptor (catecholaminergic PVT)...

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We describe 5 cases of a rare and often lethal arrhythmia: short-coupled variant of torsade de pointes. In the light of quitecharacteristic electrocardiograms and clinical picture of this arrhythmia, we consider the name 'short-coupled variant torsade de pointes' as more appropriate than, the more commonly applied term for such cases - 'idiopathic ventricular fibrillation'. We suggest that in patients with unexplained syncope, normal echocardiogram, normal QT interval and frequent premature ventricular contraction with short coupling this arrhythmogenic entity should be suspected...